Colonic lymphoma producing alpha-chain disease protein.
Alpha-chain disease with involvement of small intestine-resulting in characteristic villus atrophy and malabsorption has not been reported in this country. We studied a 57-yr-old male who presented with a polypoid tumor of the hepatic flexure of the colon. There was no evidence of malabsorption as manifested by a normal fat balance, serum carotene, and D-xylose absorption studies and the small bowel biopsy did not show villus atrophy. The tumor in the colon was surgically removed and diagnosed as a malignant lymphoma of lymphocytic type. Tumor tissue infiltrated in the mesentery could not be excised. Alpha-chain disease protein was demonstrated in serum and urine, and also in tumor tissue by immunoperoxidase techniques. The alpha-chain disease protein was further purified and classified as subclass 1. The patient had a good clinical response to cyclophosphamide and prednisone, but still has intraabdominal lymphoma with gastric involvement, and his serum alpha-chain protein persists. This case report may represent a distinct variant of alpha-chain disease.
D003110:Colonic Neoplasms / Q000150:complications* / Q000276:immunology / Q000473:pathology; D006362:Heavy Chain Disease / Q000209:etiology*; D006801:Humans; D007137:Immunoglobulin alpha-Chains; D008228:Lymphoma, Non-Hodgkin / Q000150:complications* / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged
D007137:Immunoglobulin alpha-Chains